The demand for hydrogen peroxide is booming since it is considered as one of the most environmentally friendly and versatile chemical oxidants available and has a wide range of applications. The annual market, close to 3000 kt per year being produced via the auto-oxidation process (with 2-ethyl anthraquinone (traditional) or amyl anthraquinone for mega-plants), is mostly supplied by the company Solvay (30%), followed by Evonik (20%) and Arkema (13%). Nevertheless, the dream of a direct synthesis process is close to a century old and it has gained momentum in research efforts during the last decade with more than 15 groups active in the world. In this review, we focus the discussion on the targets, e.g. plant tonnage, the reactors and the most feasible industrial operational conditions, based on our experience and point of view using the chemical engineering tools available. Thus, direct synthesis can be competitive when on-site production is required and capacities less than 10 kt per year are He has led and participated in 3 EU, 6 national/regional projects and 10+ SME contracts and has published 30+ research papers. Teresa MorenoTeresa Moreno obtained her degree in Chemical Engineering from Complutense University (Madrid, Spain) and later completed her PhD in Chemical Engineering at the University of Valladolid (Spain, 2011) studying the catalytic direct synthesis of hydrogen peroxide in supercritical CO 2 and the online determination of the product using Raman spectroscopy. She is currently a Research Scientist in the Industrial Bioactive Technologies Group at Callaghan Innovation (New Zealand) working on the development of processes for adding value to natural materials toward high value products and applications, including the development of sustainable technologies using supercritical fluids. View Article OnlineView Journal | View Issue and semi-continuous modes of operation. However, at the moment, demonstrations of continuous operations as well as carefully determined kinetics are needed in order to scale up the process. Finally, operational conditions, including the catalyst composition (active metal, oxidation state and support), promoters (halides and acids-pH-isoelectric point), solvents, pressure and temperature need to be carefully analysed. In our opinion, as we try to show here, H 2 O 2 direct synthesis is a competitive process and is ready for larger scale demonstration. Also, more than a hundred patents within the area support this claim, although the barriers of technology demonstration and further licensing are still pending.

Pisa syndrome is a rare type of truncal dystonia. Its development is associated commonly with neuroleptic treatment, but there are rare idiopathic cases or those related to neurodegenerative disorders. Recently, an association between cholinesterase inhibitors and Pisa syndrome has been described. The authors report two patients, one with Alzheimer's disease treated with risperidone and another with Parkinson's disease who presented this kind of dystonia after donepezil initiation. In the first patient the condition resolved after discontinuation of risperidone, and in the second one the condition resolved when donepezil was withdrawn. In patients with pharmacologic or degenerative dopaminergic neurotransmission disorders, cholinergic excess may induce this peculiar type of dystonia.

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Congenital muscular dystrophy type 1A (MDC1A) is caused by mutations in the LAMA2 gene encoding laminin‐α2. We describe the molecular study of 26 patients with clinical presentation, magnetic resonance imaging and/or laminin‐α2 expression in muscle, compatible with MDC1A. The combination of full genomic sequencing and complementary DNA analysis led to the particularly high mutation detection rate of 96% (50/52 disease alleles). Besides 22 undocumented polymorphisms, 18 different mutations were identified in the course of this work, 14 of which were novel. In particular, we describe the first fully characterized gross deletion in the LAMA2 gene, encompassing exon 56 (c.7750‐1713_7899‐2153del), detected in 31% of the patients. The only two missense mutations detected were found in heterozygosity with nonsense or truncating mutations in the two patients with the milder clinical presentation and a partial reduction in muscle laminin‐α2. Our results corroborate the previous few genotype/phenotype correlations in MDC1A and illustrate the importance of screening for gross rearrangements in the LAMA2 gene, which may be underestimated in the literature.

Summary:Purpose: To report on the association of childhood absence epilepsy and paroxysmal dyskinesia (PD).Methods: We describe six patients aged 6 to 27 years (mean, 14 years) who were identified in five centers participating in a European study group. Patients had been followed up clinically from the first symptoms and had been studied with video-EEG recordings of absence seizures, videotaping of dyskinetic attacks, and brain magnetic resonance imaging (MRI).Results: Four patients were sporadic, and two were siblings. Age at onset of absence seizures was unusually early (range, 3 months to 3 years 6 months; mean, 16 months), with four children having their first episodes within the first year of life, and the remaining two by age 3 years 6 months. Resistance to multiple appropriate drugs was seen in five children, in four of whom absences improved remarkably when ethosuximide was added. Absences remitted between age 8 and 13 years in the three patients in whom follow-up was long enough. Different types of PD were seen including paroxysmal kinesigenic dyskinesia (one patient), paroxysmal exercise-induced dystonia (three patients), and paroxysmal tonic upgaze (two siblings). In most patients, PD appeared at a later age than, co-occurred with, and outlasted absence seizures. Only in the two siblings with tonic upgaze, dyskinetic attacks had an earlier onset. PD improved with increasing age and did not usually produce severe disability.Conclusions: There is a widening spectrum of epilepsy and PD syndromes, within which epilepsy has the characteristics of the common idiopathic syndromes, with some atypical features.

The effects of finishing time, (T0=0, T1=30 and T2=60days), on Holstein-Friesian cull cows (n=18) and post-mortem ageing, (1, 7, 14, 21, 35 and 42days), under vacuum conditions of Longissimus thoracis (LT) muscles were investigated. The objective of this research was to study how finishing feeding (based on a commercial concentrate and corn silage), following a pasture period of 90days, affected carcass and meat quality. Ageing time effect was also evaluated on the main quality attribute of added value pieces, such as "striploin of ox" from cull cows. Finishing treatment affected intramuscular fat content (IMF), moisture percentage, water-holding capacity (WHC), colour parameters and shear force of meat at 24h post-mortem, whereas ageing time enhanced meat tenderness, when this was measured by two textural tests, Warner-Braztler (WB) and textural profile analysis (TPA). A minimum shear force was achieved at 7 and 14days of ageing for T1 and T2, respectively. No differences (P>0.05) could be found in colour parameters from 7 to 42days. The results show that a finishing time of two months is very beneficial, due to the increase in meat fatness, improved overall carcass quality and luminosity (L*). Furthermore, 14 ageing days were sufficient to improved tenderness. Ageing time did not have an effect on lipid oxidation (P>0.05) and this leads us to conclude that meat shelf life exceeded 42days under vacuum conditions'.

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The effect of weaning calves at different ages (NW=not weaned, W5=5.5 months old and W2=weaned after being born and then fed with natural Holstein Friesian milk until 2 months old), on animal performance and carcass and Longissimus thoracis muscle quality, was studied in 36 eight month-old Rubia Gallega calves. Feed ingestions, weight gains, slaughter (SW) and carcass weights (CW), carcass conformation and fat scores, and meat characteristics (24 hours postmortem): colour, pH, water holding capacity, chemical composition and texture (Warner Bratzler (WB) test, sensory panel), were studied. NW calves showed the highest SW and CW (P<0.001). Yellowness (b*), redness (a*) and chroma (C*) were higher in NW than W2 calves (P<0.05). Shear firmness was higher in W2 than in NW and W5 veal. W2 veal was less elastic (P<0.05), tender (P<0.05) and juicy (P<0.01) than NW and W5 veal.